Pediatric autoimmune neuropsychiatric disorders associated with streptococci (PANDAS)

Pediatric autoimmune neuropsychiatric disorders associated with streptococci (PANDAS)

PANDAS in short

• PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Group A Beta-Hemolytic Streptococcal Infection) is a pediatric autoimmune condition that affects a subset of children with obsessive-compulsive disorder (OCD) and/or tics, triggered by a Group A streptococcal infection.

A subset of pediatric acute-onset neuropsychiatric syndromes, resembling tic disorders and OCD, has been implicated to associate with recent GAS infection in school-aged children. These are classified as PANDAS. Working diagnostic criteria include OCD or tic disorder of abrupt onset and episodic course between 3 years of age and puberty, temporally related to GAS infection, with neurologic abnormalities during exacerbations. The pathogenesis of these disorders and role of GAS are not well characterized and somewhat controversial, although there is a high association with the B-cell alloantigen D8/17.

Synonyms

• Pediatric autoimmune diseases due to streptococcal infections
• Pediatric neuropsychiatric autoimmune diseases due to streptococcal infections

How common is PANDAS?

• Its exact prevalence is unknown, but an estimated 10–25% of children with OCD—affecting about 2% of the general population—may meet the diagnostic criteria for PANDAS.
• The condition shows a male-to-female ratio of approximately 2.6:1.

Age of onset: Childhood

What causes this condition?

• The exact cause remains unclear. One proposed mechanism suggests that streptococcal infections trigger an immune response in certain children, leading to inflammation or dysfunction in the basal ganglia.
• No specific genetic basis has been established.

What are the symptoms of PANDAS?

• Diagnostic criteria include: the presence of OCD and/or tics, symptom onset before puberty, a sudden and dramatic onset of symptoms, and a clear temporal relationship between streptococcal infections and the remission-relapse pattern of neuropsychiatric symptoms such as motor hyperactivity or involuntary movements, including chorea-like movements or tics.

Very frequent symptoms

HP Tics 

Frequent symptoms

• Abnormal fear/anxiety-related behavior
• Attention deficit hyperactivity disorder
• Chorea 
• Depression
• Developmental regression 
• Emotional lability
• Impulsiveness
• Irritability
• Obsessive-compulsive trait
• Oppositional defiant disorder
• Personality changes
• Sleep abnormality

Occasional symptoms

• Agoraphobia
• Anorexia
• Arthralgia
• Claustrophobia
• Clumsiness
• Encopresis
• Enuresis
• Phonophobia
• Recurrent streptococcus pneumoniae infections
• Separation insecurity

Symptom worsening in PANDAS typically lasts for several weeks and may persist for months or longer, followed by a gradual and progressive improvement.

A key characteristic of the disorder is the transient link between neuropsychiatric symptom flare-ups and streptococcal infections.

Additional neuropsychiatric features are common, including emotional instability, separation anxiety, anorexia, impulsivity, attention deficits, and motor hyperactivity resembling attention deficit hyperactivity disorder (ADHD). Comorbid conditions frequently reported include depression (36%), dysthymia (6%), and separation anxiety (20%).

How is this condition diagnosed?

Diagnosis is primarily clinical, though MRI may show basal ganglia enlargement.

How is PANDAS treated?

Treatment follows standard approaches for OCD and tic disorders, including cognitive-behavioral therapy, pharmacologic interventions (such as neuroleptics), antibiotics to prevent streptococcal infections, and intravenous immunoglobulin (IVIG) in some cases.

How are Pediatric autoimmune neuropsychiatric disorders associated with streptococci treated?

Controversy also exists surrounding management, particularly the role of long-term prophylactic antibiotics and immune-modulating therapy, although intravenous immunoglobulin has been suggested as an effective treatment in some literature. Acute treatment with GAS-directed antibiotics may be reasonable, although no randomized trials exist. Otherwise, symptomatic treatment of neuropsychiatric symptoms is indicated.